基于美国儿童肿瘤学组方案治疗68例神经母细胞瘤的中长期随访结局

目的基于美国儿童肿瘤学组(COG)危险度分级的化疗方案,探讨神经母细胞瘤(NB)患儿诊断年龄与预后的相关性。方法以2007年1月至2013年3月复旦大学附属儿科医院外科诊断为NB的连续病例为研究对象,划分低危、中危和高危,分析不同INSS分期NB患儿的5年总体生存率和5年无事件生存率(EFS),并对诊断年龄和5年EFS行受试者工作特征(ROC)曲线分析,获得与EFS相关的最佳诊断年龄界值。结果 68例NB患儿进入分析,男41例(60.3%),女27例;Ⅰ期7例(10.3%),Ⅱ期14例(20.6%),Ⅲ期11例(16.2%),Ⅳ期23例(33.8%),Ⅳs期13例(19.1%)。低危24例,中危14例,高危30例。128例高危患儿术前行诱导化疗,非常好的部分缓解率为60.7%(17/28),部分缓解率为14.3%。60/68例接受手术治疗,肉眼完整切除(GTR)率为71.7%。28例失访,32例随访至5年,5年总体生存率为65.6%,其中Ⅲ期为66.7%,Ⅳ期为22.2%;5年EFS为59.4%,其中Ⅲ期为50.0%,Ⅳ期为11.1%,GTR患儿5年EFS高于未GTR患儿(70.2%vs 57.4%)。35/30例MYCN基因扩增阳性(Ⅱ期1例,Ⅳ期4例),2年总体生存率为40%(2/5),2年EFS为20%(1/5);25例MYCN基因阴性患儿,2年总体生存率为92%(23/25),2年EFS为88%(22/25)。4诊断年龄和5年EFS的ROC曲线下面积为0.713,诊断年龄2.4岁时的敏感度为75.0%,特异度为66.7%。结论采用COG治疗方案的Ⅳ期患儿的5年总体生存率为22.2%,5年EFS为11.1%。GTR与NB的预后相关,诊断年龄2.4岁可能提示预后不良。     

小儿再生障碍性贫血疗效总结

目的总结72例小儿再生障碍性贫血(以下简称再障)的疗效。方法72例患儿中30例慢性再障,42例急性再障。慢性再障给予安雄、CsA治疗,急性再障给予安雄、CsA、大剂量丙种球蛋白、ATG治疗,其中2例接受异基因造血干细胞移植。结果72例再障患儿中慢性再障组和急性再障组在性别和发病年龄上无明显差异(P>0.05)。30例慢性再障病人中17例进步或治愈,11例无效或进展,无死亡病例;42例急性再障病人中14例进步或治愈,20例无效或进展,7例死亡,两组病人达进步或治愈者均无复发病例,两组总有效率有显著差异(56.67%vs33.33%P<0.05),两组病人总的生存率统计学上有差异(100%vs83.33%P<0.05)。两组出现疗效的时间无差异(P>0.05),而在中位输血时间间隔上,两组有显著差异(P<0.005)。在42例急性再障病人中,28例接受ATG治疗,14例未接受ATG治疗,ATG组中有11例出现进步或治愈,未接受ATG组中仅3例出现疗效,两组有明显统计学差异(42.13%vs21.43%P<0.05),72例再障病人中有3例转变为AML(分别为M2,M3,M5),2例接受了异基因造血干细胞移植后治愈。结论慢性再障治疗疗效及生存率明显高于急性再障,而联合免疫治疗是治疗急性再障除了骨髓移植外的最有效的方法。     

The Assessment of Preschool Children's Participation: Internal Consistency and Construct Validity

ABSTRACT

Participation in activities provides the means for young children to learn, play, develop skills, and develop a sense of personal identity. The Assessment of Preschool Children's Participation (APCP) is a newly developed measure to capture the participation of children aged 2 to 5 years and 11 months in the areas of play, skill development, active physical recreation, and social activities. Data from a clinical trial involving 120 children with cerebral palsy indicated that the APCP has moderate to very good internal consistency. The measure distinguishes between children below or above 4 years of age across levels of the Gross Motor Classification System, and between income levels below or above the median regional income range. The APCP, with a focus on preschool children, has potential use for assessment and identification of activity areas in which the child is participating and areas in which participation may be restricted.     

The Discrimination of Children's Phobias Using the Revised Fear Survey Schedule for Children

The discriminant validity of children's and parents' ratings of the child's fear on the Revised Fear Survey Schedule for Children (FSSC-R; Ollendick, 1983) was examined using a clinic sample of children who met DSM criteria for phobic disorders (N = 120). Discriminant function analyses and item analyses were conducted to determine if children meeting diagnostic criteria for a primary disorder of social phobia, simple phobia of the dark/sleeping alone, simple phobia of animals, or a simple phobia of shots/doctors could be differentiated on the basis of FSSC-R subscale scores and items. Results of the discriminant function analyses indicated that the child-completed as well as the parent-completed FSSC-Rs were similarly useful in differentiating the specific types of phobias. Results of the item analyses indicated that child-completed FSSC-R items could discriminate among the different simple phobias but not social phobia and that parent-completed FSSC-R items could discriminate not only the different simple phobias but also social phobia. Results are discussed in terms of the utility of the FSSC-R in the assessment of clinically significant fears in children with phobic disorders and the relative utility of child and parent ratings in the assessment of childhood fears.     

儿童骨肉瘤单中心诊治分析:附25例报告

目的制定本中心儿童骨肉瘤治疗方案,对该方案进行疗效分析。方法前瞻性制定包括病理分型、临床分期、分组、化疗、手术的整体性诊断治疗方案,方案适用对象为1998年10月-2009年12月期间经病理检查确诊为骨肉瘤的连续初治病例,共25例,按方案根据不同个体对治疗反应的差异在不同治疗阶段手术,按影像学和病理学对治疗反应的评估,给予不同强度的化疗。分析本组患儿的治疗依从性和远期预后。结果全组25例,放弃治疗1例,接受手术10例,完成术前化疗2～4个疗程后拒绝手术14例。完成手术及化疗计划8例,其中4例无事件生存>32个月;2例手术者在术后化疗中复发。未手术者14例中3例在2～36个月内重新进展,12例失访。结论儿童骨肉瘤治疗依从性差,局部病灶根治性手术率低是影响预后的最重要因素。     

动脉化疗栓塞术对小儿肾母细胞瘤细胞凋亡和增殖的影响

目的探讨动脉化疗栓塞术(TACE)对肾母细胞瘤(WT)细胞凋亡指数(AI)和细胞增殖指数(PI)的影响,并评价其在临床治疗中的地位。方法将24例肾母细胞瘤患儿按临床分期、病理分型进行配对设计研究,设动脉化疗栓塞组12例,全身化疗组12例,采用TUNEL法和免疫组织化学超敏二步法测定标本的细胞凋亡指数和细胞增殖指数。结果动脉化疗栓塞组AI高于全身化疗组(P<0.05),PI低于全身化疗组(P<0.05),AI与PI成负相关(P<0.05),动脉化疗栓塞组术后瘤体缩小,坏死程度高于全身化疗组(P<0.05),而骨髓抑制少(P<0.05)。结论与全身化疗组相对比,术前采用动脉化疗栓塞法能更有效地诱导肿瘤细胞凋亡,抑制肿瘤细胞增殖,促使肿瘤缩小、坏死,有利于手术根治,取得更好的疗效,提高生存率。     

2009至2010年西安市儿童医院急诊患儿死亡原因分析

目的 了解近2年西安市儿童医院急诊患者死亡疾病谱变化和直接死亡原因.方法 对2009年至2010年我院收治的130例急诊死亡病例进行回顾性分析.结果 2009年主要死亡疾病依次为:肺炎,先天性心脏病,窒息,新生儿肺出血,颅内出血;2010年主要死亡疾病依次为:肺炎,窒息,病毒性脑炎,先天性心脏病,新生儿肺出血,两年死亡疾病谱发生了明显变化.91例患儿找到直接死亡原因,依次为:呼吸衰竭25例(27.5％),心力衰竭17例(18.7％),休克17例(18.7％),窒息16例(17.5),脑疝16例(17.5％).结论 要降低急诊患儿的病死率,必须加强婴幼儿卫生保健,积极治疗肺部感染和全身感染,积极抗休克治疗,控制心力衰竭,以及预防误吸.     

Correlation of Obesity and Overweight with Emotional-Behavioral Problems in Primary School Age Girls in Tabriz,Iran

Objective

Obesity and overweight have shown an increasing trend in most developing countries. Childhood obesity would impose numerous health-related problems. This study was conducted to determine the correlation of obesity and overweight with emotional-behavioral problems in primary school age girls.

Methods

In a cross-sectional study, 300 primary school girls (aged 7-11 years) were selected using a multi-staged sampling method, including randomized cluster and stratified method. For all students body mass index was measured and then based on BMI for age and sex, from each grade (1-5), 20 students were selected for each group of normal weight, overweight and obese. The emotional-behavioral problems were evaluated using child behavior checklist that consists of 113 items for childhood behavioral problems.

Findings

Total behavioral problems were seen in 17%, 27%, and 2% in obese, overweight, and normal weight children, respectively. Internalizing problems (including Anxious/Depressed, Withdrawn, and Somatic Complaints) were seen in 11%, 15%, and 2% and externalizing problems (including Aggressive and Delinquent Behaviors) were observed in 8%, 17%, and 2% in obese, overweight, and normal weight children, respectively. The mean scores in all scales were higher in obese and overweight children in comparison with normal weight children and the emotional-behavioral problems had significant positive correlation with obesity and overweight (P<0.01).

Conclusion

Despite the cultural differences between east and west, yet there are similarities in the most of the emotional-behavioral problems related to overweight and obesity. Also, it seems that the risk of behavioral-emotional problems in overweight girls is more than in obese girls. Overweight and Obesity prevention may be a primary preventive step for these problems in children.     

术前化疗对神经母细胞瘤细胞凋亡和增殖的影响作用

目的：探讨术前化疗对神经母细胞瘤细胞凋亡和肿瘤增殖的作用。方法：应用脱氧核糖核酸末端转移酶标记（ＴＵＮＥＬ）和增殖细胞核抗原（ＰＣＮＡ）免疫组化，观察术前化疗组６例和一期手术组１１例神经母细胞瘤标本的凋亡指数、增殖指数和分裂指数的差异。结果：术前化疗组凋亡指数（９．１７±４．８８）与一期手术组（１０．１１±５．１６）无显著差异，Ｐ＞０．０５；术前化疗组增殖指数（１８．００±９．５５）明显低于一期手术组（２９．００±１３．８０），术前化疗组的分裂指数（１．６７±１．３４）亦较一期手术组（３．６７±２．２４）有明显下降，Ｐ值均＜０．０５。结论：术前化疗对神经母细胞瘤的肿瘤细胞增殖有明显抑制作用。ＴＵＮＥＬ和ＰＣＮＡ检测为观察肿瘤细胞凋亡和细胞增殖的有效方法     

A Group Motor Skills Program for Children with Coordination Difficulties: Effect on Fundamental Movement Skills and Physical Activity Participation

Aims: Children with coordination difficulties are at risk of low levels of physical activity (PA) participation. This intervention examined the effects of a multidisciplinary program that emphasized parent participation on motor skill performance and PA. Methods: Ten boys (5–7 years) completed a group program consisting of conditioning exercises and activities designed to address child-selected goals. Motor proficiency and PA participation were assessed before and after the program using the Test of Gross Motor Development (TGMD-2) and triaxial accelerometers, respectively. Rating scales captured child and parent perceptions of performance for each child's goals. Results: TGMD-2 subtest raw scores, age equivalent and percentile scores improved, along with parent ratings of their child's performance. Six children reported skill improvements. On average, moderate to vigorous PA improved by 10 min per day although these gains were not significant. Time spent in sedentary activities was unchanged. None of the children met the Canadian PA and sedentary behaviour guidelines. Conclusions: The results support effectiveness of a group program to improve gross motor performance and levels of PA in children with coordination difficulties. Gains in both of these domains also have the potential to impact quality of life and reduce health risks associated with inactivity.     

Effect of Age on QT Variability

Heart rate variability is influenced significantly by age. Recent studies have shown the utility of QT variability to study temporal repolarization lability, and increased QT variability is reportedly associated with sudden death. Because beat-to-beat QT interval variability appears to closely follow heart rate variability, this study investigated the relationship between age and QT variability. We obtained QT variability measures in 34 adults (22–55 years of age) and 15 children (6–14 years of age) using a novel algorithm to compute beat-to-beat QT intervals. QT variability corrected for mean QT interval was significantly inversely correlated with age. Coherence in the band of 0 to 0.5 Hz and high-frequency coherence (0.15–0.5 Hz) were also significantly inversely correlated with age. QT_vi (QT variability corrected for mean QT interval over heart rate variability corrected for mean heart rate) was not significantly related to age in supine posture during spontaneous or controlled breathing, whereas there was a modest negative relationship in standing posture. These findings suggest that coherence between QT and heart rate time series appears to be modulated by cardiac vagal activity.     

TREATMENT OF STANDARD-RISK ACUTE LYMPHOBLASTIC LEUKEMIA IN CHILDREN: The Results of Protocol AL841 from the Kyushu-Yamaguchi Children's Cancer Study Group in Japan

A total of 62 patients with standard-risk acute lymphoblastic leukemia received three-drug induction consisting of vincristine, prednisolone, and L-asparaginase (l-Asp) followed by consolidation therapy with intermediate-dose methotrexate (MTX), intrathecal MTX, and 18 Gy of cranial irradiation. Maintenance therapy consisting of 6 drugs including daunorubicin (DNR, 450 mg/m2 in total) was continued for 3 years. Patients were randomized and half of them received weekly l-Asp during maintenance therapy as a late intensification. Complete remission (CR) was achieved in 61/62 (98.4%), and 11 of 61 patients relapsed. At 10 years, the event-free survival (EFS) was 80.6 5.0% and overall survival was 88.7 4.0%; median follow-up time was 9.3 years. The 10-year EFS of patients with additional l-Asp (84.8 6.2%) was superior to that without l-Asp (75.9 7.9%), although it was not statistically significant. No patients who received a full dose of DNR and maintained CR developed heart failure, although the shortening fraction decreased from 41.0% at diagnosis to 35.2% (median). The protocol AL841 provided good long-term disease control without severe late cardiac dysfunction.     

Plasma Concentrations of N-Terminal Brain Natriuretic Peptide in Healthy Children, Adolescents, and Young Adults: Effect of Age and Gender

Children with congenital heart disease need adequate diagnostic classification regarding their cardiovascular status (CVS). N-terminal brain natriuretic peptide (N-BNP) plasma concentration indicates dysfunction of the cardiovascular system and guides decisions concerning treatment and prognosis. Reference values are established for adults, with age-dependent increasing values and higher values in women. To avoid misclassification concerning the CVS, a large group of healthy children and adolescents can be used show the relationship between gender, age, and N-BNP and these can serve as reference values. N-BNP was measured in 434 healthy subjects (240 female and 194 male) with ages ranging from 0 to 32 years without any cardiovascular disease or renal or hepatic impairment. Measurements were performed with an electrochemiluminescence immunoassay from Roche Diagnostics. Mean N-BNP decreased from 12.6 fmol/ml (0–9 years; n = 79) to 9.41 fmol/ml (10–14 years; n = 154) and in adolescents from 6.1 (15–19 years; n = 99) to 4.8 fmol/ml (>19 years; n = 102) in adults (p < 0.05). Mean N-BNP concerning gender did not differ in any age group younger than 19 years. In contrast, the adult female group had 78% higher N-BNP compared to the male group (p < 0.05). There was a significant peak in N-BNP at the age of 12–14 years. This study shows that reference values for N-BNP differed profoundly in children compared to adults and were up to 260% higher in children without any gender difference. Therefore, these reference values will help to avoid CVS misclassification in children for the biomarker N-BNP.     

Age is of influence on midazolam requirements in a paediatric intensive care unit

AIM: To test that age is of influence on midazolam requirements during prolonged mechanical ventilation in critically ill children. METHODS: Retrospective observational study of children (28 days-18 year) admitted between January 1st 2002 and January 1st 2005 who needed controlled mechanical ventilation for 5 days and initial sedation with midazolam were included. Exclusion criteria were psychomotor retardation, therapeutic use of midazolam, ventilator weaning within 5 days, kidney or liver failure. RESULTS: A total of 1186 children were admitted, of which 58 children were included. The children were divided into three age groups: 28 days-1 year (n = 28), 1-4 years (n = 16) and older than 4 years (n = 14). Within 2 days the children age 1-4 years received the maximum midazolam dosage (0.3 mg/kg/h). In addition, the mean total dose of midazolam was higher at all days for this age group. At day 5 none of the children between 1 and 4 years could be sedated with midazolam alone. CONCLUSIONS: Our data showed that children between 1 and 4 years needed higher doses of midazolam as compared to children who were younger and older. Furthermore, we observed that midazolam alone is a poor sedative for all age groups. The influence of and mechanisms for possible age related effects on midazolam requirements remain to be elucidated, as well as the position of midazolam as a first line drug for PICU sedation.